由欧洲皮肤病与性病学会（EADV）发起的寻常型天疱疮和落叶型天疱疮管理的S2K指南更新版。
Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV).
作者信息
Joly P, Horvath B, Patsatsi Α, Uzun S, Bech R, Beissert S, Bergman R, Bernard P, Borradori L, Caproni M, Caux F, Cianchini G, Daneshpazhooh M, De D, Dmochowski M, Drenovska K, Ehrchen J, Feliciani C, Goebeler M, Groves R, Guenther C, Hofmann S, Ioannides D, Kowalewski C, Ludwig R, Lim Y L, Marinovic B, Marzano A V, Mascaró J M, Mimouni D, Murrell D F, Pincelli C, Squarcioni C P, Sárdy M, Setterfield J, Sprecher E, Vassileva S, Wozniak K, Yayli S, Zambruno G, Zillikens D, Hertl M, Schmidt E
机构信息
Department of Dermatology, Rouen University Hospital and INSERM U905, Centre de référence des maladies bulleuses autoimmunes, Normandie University, Rouen, France.
Department of Dermatology, University Medical Center Groningen, Center for Blistering Diseases, University of Groningen, Groningen, the Netherlands.
出版信息
J Eur Acad Dermatol Venereol. 2020 Sep;34(9):1900-1913. doi: 10.1111/jdv.16752. Epub 2020 Aug 24.
BACKGROUND
Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin. Before the era of immunosuppressive treatment, pemphigus was almost always fatal. Due to its rarity, only few randomized controlled therapeutic trials are available. Recently, rituximab has been approved as first-line treatment for moderate and severe pemphigus vulgaris in Europe and the United States.
OBJECTIVES
The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology (EADV) has initiated a throughout update of the guideline for the management of patients with pemphigus.
RESULTS
The guidelines for the management of pemphigus were updated, and the degree of consent among all task force members was included. The final version of the guideline was consented by the European Dermatology Forum (EDF) and several patient organizations.
背景
天疱疮是一组危及生命的自身免疫性大疱性疾病，其特征为黏膜和皮肤出现水疱及糜烂。在免疫抑制治疗时代之前，天疱疮几乎总是致命的。由于其罕见性，仅有少数随机对照治疗试验。最近，利妥昔单抗在欧洲和美国已被批准作为中度和重度寻常型天疱疮的一线治疗药物。
目的
欧洲皮肤病与性病学会（EADV）自身免疫性大疱性疾病特别工作组已着手全面更新天疱疮患者管理指南。
结果
天疱疮管理指南得到更新，并纳入了所有特别工作组成员的同意程度。该指南的最终版本得到了欧洲皮肤病学论坛（EDF）和几个患者组织的认可。
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